Posterior urethral valves - Great Ormond Street Hospital

Great Ormond Street Hospital for Children NHS Foundation Trust: Information for Families
Posterior urethral valves
This leaflet aims to explain about posterior urethral
valves (PUV) and what to expect when your child comes
to Great Ormond Street Hospital (GOSH) for treatment.
How does the urinary
system work?
What are
posterior urethral valves?
The urinary system consists of the kidneys,
the bladder and ureters. The kidneys
filter the blood to remove waste products
and form urine. The urine flows from
the kidneys down through the ureters to
the bladder. During urination it passes
through another tube called the urethra
to the outside when urinating (peeing).
Posterior urethral valves (PUV) is a
condition found only in boys. It affects
the urethra (the tube which runs from
the bladder to the outside). In boys with
PUV, the urethra has a blockage in it near
the bladder. This makes it difficult for
your son to pass urine. As the bladder
pushes hard to get the urine out, it
causes pressure which may result in urine
being pushed back from the bladder into
the ureters and kidneys. This causes the
kidneys and bladder to swell and may
lead to kidney damage.
What are the
symptoms of PUV?
kidneys
There are various symptoms associated
with PUV, but they may not affect every
child in the same way. The degree of
blockage affects the severity of the
symptoms. Some symptoms include:
ureters
„„an enlarged bladder, so that it can be
felt through the abdomen as a lump
„„urinary tract infections (UTIs)
bladder
„„difficulty urinating
„„a weak stream of urine
sphincter
urethra
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„„unusually frequent urination
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© GOSH NHS Foundation Trust November 2012
„„bed wetting after toilet training has
been successful
„„poor weight gain
However, these symptoms can resemble
those of other conditions, so you should
always check with your doctor.
„„Blood tests – these will show how well
your child’s kidneys are working and
check that there is enough fluid inside
the body. Other tests and scans may be
needed to check that other parts of the
urinary system are working properly.
These can include kidney and bladder
function tests.
How is it diagnosed?
PUV can be diagnosed by a routine
ultrasound scan during pregnancy if the
bladder, ureters or kidneys are swollen.
It can also be diagnosed in a newborn
baby if the bladder is swollen and urine
dribbles constantly. If the blockage was
not severe before or just after birth, the
condition can remain undetected until the
child has symptoms as above.
What causes PUV?
The severity of the blockage affects how
the condition is diagnosed. Each case will
be different, but the following tests are
usual:
How rare is it and
who is affected?
„„Ultrasound of your son’s abdomen
– this is very similar to the ultrasound
scan that most women have during
pregnancy. It creates a picture of the
organs inside your son’s body and
shows how well they are working.
„„Micturating cystourethrogram
(MCUG) – this test shows urine passing
from the bladder to the urethra and
then to the outside. It will also show if
the urine is flowing backwards towards
the ureters and kidneys (this is known
as reflux).
„„Cystoscopy – this is a scan which uses
a small tube with a camera at the end
to examine the inside of the bladder.
For more information about this test,
please see our Cystoscopy leaflet.
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It is not inherited in any recognised way
and seems to happen in the early stages
of pregnancy when the organs, muscle
and other tissue starts to form. It is not
due to anything you did or ate during
pregnancy.
PUV seems to occur in one in every 8000
births and affects only boys.
How can PUV be treated?
There are several options for treatment,
depending on how severely the symptoms
are affecting your child:
„„The first course of action usually deals
with the symptoms and includes putting
a catheter (thin, plastic tube) into the
bladder to drain away the urine. Any
UTIs and fluid imbalance will also be
treated with antibiotics and intravenous
(IV) fluids, which are delivered directly
into a vein.
„„The next course of action is to try to
remove the parts of the valve which are
causing the backflow of urine into the
kidneys. This is usually carried out using
© GOSH NHS Foundation Trust November 2012
a cystoscope (a tube with a light on the
end) rather than a traditional ‘open’
operation.
What is the outlook
for children with PUV?
The outlook depends on how much
damage has already occurred in the
kidneys. Your doctors will be able to
give you a better idea once they have all
of your son’s test and blood results. Your
son will need follow up appointments
on a long term basis to check that no
lasting damage has been done to the
urinary system.
Is there a support group
for people with PUV?
No there is no support group in the UK
which helps people with PUV, but the
following organisation may be able to
put you in touch with another family in a
similar situation:
Contact a Family
Helpline: 0808 808 3555
Email: [email protected]
Website: www.cafamily.org.uk
If you have any questions, please call Squirrel Ward on 020 7829 8814
Notes
Compiled by the depatment of Urology in collaboration with the Child and Family Information Group
Great Ormond Street Hospital for Children NHS Foundation Trust
Great Ormond Street,
London WC1N 3JH
www.gosh.nhs.uk
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Ref: 2012F0568
© GOSH NHS Foundation Trust November 2012